Hurler-Scheie syndrome

Hur·ler-Scheie syn·drome

(hŭr'ler shā),
a phenotypic intermediate between Hurler syndrome and Scheie syndrome; a deficiency of α-l-iduronidase.
[Gertrud Hurler, Harold G. Scheie]

Hurler-Scheie syndrome

(hûr′lər-shī′)
n.
A type of mucopolysaccharidosis (MPS 1H/S) characterized by symptoms that are intermediate in severity between those of Hurler syndrome and Scheie syndrome.

Hur·ler-Scheie syn·drome

(hŭrlĕr shā sindrōm)
Phenotypic intermediate between Hurler syndrome and Scheie syndrome.
[Gertrud Hurler, Harold G. Scheie]
References in periodicals archive ?
Patients with Hurler-Scheie syndrome may suffer from mild cognitive impairment or problems with attention.
Deficiency of this enzyme results into a wide range of phenotypes including Hurler syndrome, Hurler-Scheie syndrome (OMIM 607015) and Scheie syndrome (OMIM 607016), severe, intermediate and mild forms, respectively (3).
Cambridge, MA) announced the issuance of a third United States patent involving the lysosomal enzyme alpha-L-iduronidase (IDUA), which is deficient in persons suffering from mucopolysaccharidosis type I (MPS I), also referred to as Hurler syndrome, Hurler-Scheie syndrome, or Scheie syndrome.
US-based biotechnology company ArmaGen, Inc has reported preliminary evidence of cognitive improvement in children treated with AGT-181, the company's investigational therapy for the treatment of Hurler and Hurler-Scheie syndrome (also known as mucopolysaccharidosis type I, or MPS I), the company said.
12) could determine [alpha]-Liduronidase activity in DBS and thus identify patients with mucopolysaccharidosis type I (MPS-I), representing all three clinical subtypes of Hurler, Scheie, and Hurler-Scheie syndromes.